The association of
sarcoidosis and
immune thrombocytopenia (
ITP) has rarely been investigated. The aim of the current retrospective study was to investigate the clinical and biological phenotypes and outcome of this association in a large series of recent patients. Twenty patients (50% men) were included. Median age at
sarcoidosis and
ITP diagnosis was 36 (range, 10-83 yr) and 38 (range, 21-83 yr) years, respectively. In 11 of 20 (55%) patients,
sarcoidosis onset preceded
ITP (median interval, 48 mo; range, 6-216 mo). In 5 of 20 (25%) patients, the 2 conditions occurred concomitantly. In 4 of 20 (20%) patients,
ITP onset preceded
sarcoidosis (median interval, 68 mo; range, 15-153 mo). In 4 cases,
sarcoidosis and
ITP were not concomitant, since 1 condition was cured before the other was declared. In 12 of 20 (60%) patients there was a simultaneous onset or relapse of both
ITP and
sarcoidosis.
Sarcoidosis phenotype was characterized by an acute onset in 40% of patients. The visceral involvement included thoracic sites in 19 of 20 (95%) patients and extrathoracic sites in 16 of 20 (80%) patients. At
ITP onset, median platelet count was 11 × 10/L (range, 3-90); 17 (85%) patients had a platelet count <30 × 10/L. Seven (35%) patients had a
bleeding score >8 without visceral
bleeding.Nineteen of the 20 (95%) patients were treated specifically for
ITP. After the first-line
therapy (
prednisone at 1 mg/kg per day for at least 3 consecutive weeks in all patients; with
IVIg in addition for 10 patients with severe
bleeding score), 12 of 19 (63%) patients achieved a complete response, 6 (31.5%) had a partial response, and only 1 patient failed to respond. At the end of
ITP follow-up (median, 70 mo; range, 12-142 mo), 18 (90%) patients achieved a complete response, 1 achieved a partial response, and 1 had no response. After a median follow-up of 105 months, 13 of 20 (65%) patients had persistent
sarcoidosis requiring prolonged
therapy, and thus
sarcoidosis represented the main long-term concern. Main conclusions were 1)
ITP presentation was usually severe, but response to treatment was favorable in almost all cases, with no death and no severe
bleeding, in contrast with older reports, 2)
sarcoidosis was remarkable for the high proportion of cases with an acute onset, a chronic course, and the need for prolonged
prednisone therapy, 3)
sarcoidosis and
ITP onset and evolution were not always synchronous.